eISSN: 2299-0038
ISSN: 1643-8876
Menopause Review/Przegląd Menopauzalny
Current issue Archive Manuscripts accepted About the journal Special Issues Editorial board Abstracting and indexing Subscription Contact Instructions for authors Publication charge Ethical standards and procedures
Editorial System
Submit your Manuscript
SCImago Journal & Country Rank


2/2023
vol. 22
 
Share:
Share:
Case report

A rare case of angiomyomatous hamartoma in the pelvic and paraaortic lymph nodes

Angel Yordanov
1
,
Tatyana Betova
2
,
Savelina Popovska
2
,
Stoyan Kostov
3
,
Yavor Kornovski
3
,
Yonka Ivanova
3
,
Stanislav Slavchev
3
,
Ilko Iliev
1
,
Venelina Todorova
4

  1. Department of Gynaecological Oncology, Medical University Pleven, Pleven, Bulgaria
  2. Department of General and Clinical Pathology, University Hospital “Dr. Georgi Stranski,” Pleven, Bulgaria
  3. Department of Gynecology, St. Anna University Hospital, Medical University-Varna “Prof. Dr. Paraskev Stoyanov”, Varna, Bulgaria
  4. Imaging Department, University Hospital “Dr. Georgi Stranski,” Pleven, Bulgaria
Menopause Rev 2023; 22(2): 111-116
Online publish date: 2023/06/14
Article file
- A rare case (1).pdf  [0.32 MB]
Get citation
 
PlumX metrics:
 

Introduction

Angiomyomatous hamartoma of the lymph node (AMH-LN) is an extremely rare, benign vascular disease of unknown etiology [1], which is characterized by partial or complete replacement of the lymph node parenchyma by irregularly distributed thick-walled blood vessels, smooth muscle bundles and adipose tissue in a fibrotic stroma [2]. It was described for the first time by Chan et al. in 1992 [3], and to our knowledge, there are 70 cases described in the literature to date. Angiomyomatous hamartoma (AMH) occurs mainly in inguinal and femoral nodal regions, but there are a few reports of some other locations – submandibular, cervical, popliteal and paraaortic lymph nodes [48]. We present a case of a 37-year-old female patient with AMH-LN in the pelvic and paraaortic lymph nodes who presented with weight loss.

Case report

A 37-year-old woman was referred to our clinic due to weight loss – 7 kg in 7 months, moderate pelvic pain syndrome and suspected pelvic tumor. Upon admission to the Gynaecologic Oncology Department, her weight was 39 kg, and BMI was 16.23. The patient’s medical history record showed bronchial asthma and two previous Cesarean section – in 2007 and 2014. The laboratory tests and serum tumor marker (Ca-125) were normal. There was no evidence of acute or chronic inflammatory disease. The gynecological examination of the pelvis revealed a soft, poorly mobile tumor with unclear borders measuring 5/5 cm. It was assumed to be an ovarian tumor on the left. Vaginal ultrasound was performed: a normal uterus and adnexa were visualized; an echo-homogeneous tumor on the right, with smooth external and internal walls measuring 40/45 mm, was found. Computed tomography (CT) scan of the pelvis and abdomen was performed.

Computed tomography revealed numerous pathologically enlarged bulky lymph nodes in the paracaval and paraaortic area in size up to 44/109 mm (Fig. 1 A, B). Bulky lymph nodes on the right, paralytic, with sizes up to 37/24 mm, were detected. Bulky lymph nodes parallel to the left with 38.4/44.4 mm dimensions were visualized (Fig. 1 C, D).

Fig. 1

Computed tomography images before and 6 months after the surgical procedure. A) Paraaortic lymph nodes in the thoracic segment before surgery; B) bilaterally paraaortic lymph nodes in clusters at the level of the renal vessels before surgery; C) left parailiac lymph nodes before surgery; D) right parailiac lymph nodes before surgery; E) paraaortic lymph nodes in the thoracic segment after surgery; F) bilaterally paraaortic lymph nodes in clusters at the level of the renal vessels after surgery; G) left parailiac lymph nodes after surgery; H) right parailiac lymph nodes after surgery

/f/fulltexts/PM/50830/MR-22-50830-g001_min.jpg

A decision for surgical treatment was made in order to remove the described tumor and biopsy of the enlarged lymph nodes.

After standard preoperative preparation, a lower middle laparotomy was performed. Normal uterus and adnexa were visualized. Retroperitoneally on the left, a soft suspected enlarged lymph node with unclear boundaries was palpating, reaching the level of the bifurcation of the left common iliac artery with a size of 10/10 cm, which could not be differentiated by the pelvic vessels. This suspected enlarged lymph node was removed, leaking chylous contents. A lymphoproliferative process was intraoperatively diagnosed, which necessitated the completion of the intervention.

The material was represented by a poorly defined mass with partially preserved lymphoid tissue, replaced mainly by muscle-type vascular structures with pronounced smooth muscle hyperplasia in the wall, atrophic lymph follicles and mature adipose tissue mixed in different proportions, but with a predominance of the vascular component (Fig. 2 A). The latter is represented by channels covered by a single endothelium and filled with erythrocytes and fibrin. In immunohistochemical examination, the smooth muscle cells showed a diffuse positive reaction for smooth muscle actin (Fig. 2 B), positive expression for CD31 was observed in endothelial cells (Fig. 2 C), and the adipose tissue showed a positive reaction for S100 (Fig. 2 D). A negative reaction was reported for HMB-45, melan A, and a slightly positive reaction for estrogen. No necrosis, cytological atypia or mitosis was detected. Outside the compact vascular component, the blood vessels were deformed with hypertrophied and hyalinized media.

Fig. 2

Histological findings. A) Angiomyomatous hamartoma (AMH) – haphazardly arranged, fully formed, thick-walled, muscular blood vessels, surrounded by smooth muscle cells and fibrous stroma, occasional focal normal adipose tissue, and atrophic lymphoid tissue, HE 100×; B) AMH – smooth muscle actin immunostaining reveals the presence of numerous smooth muscles in the blood vessel wall and intervening spaces, immunohistochemistry – IHC, 100×; C) AMH – CD31 immunostaining stains the vascular endothelial cells and highlights the rich vascularity of the lesion, IHC 100×; D) AMH – S100 immunostaining expression fat component, IHC 100×

/f/fulltexts/PM/50830/MR-22-50830-g002_min.jpg

A benign lesion closest to AMH of the iliac lymph nodes on the left, a variant of AMH, was established based on morphology and immunohistochemistry examination.

The patient was provided with an abdominal drain for 48 hours and was discharged on the 5th postoperative day in good health condition.

At a follow-up examination 3 months later, the patient had regained 6 kg of her weight with a BMI of 18.73 and reported no pain syndrome. Six months after the operation, follow-up CT of the pelvis and abdomen was performed. The CT revealed persistence of the described paraaortic lymph nodes with a slight increase in size (Fig. 1 E, F), absence of tumor in the area of the left pelvic lymph nodes (Fig. 1 G) and persistence without resizing the right pelvic lymph nodes on the right (Fig. 1 H).

Discussion

Angiomyomatous hamartoma of the lymph node is a rare, benign disease that, according to the literature, affects men more often than women in a ratio of 2 : 1 [9]. Our search does not confirm the data. In the literature, we found 38 cases of men and 31 of women, including the present case. Angiomyomatous hamartoma of the lymph node most often involves the inguinal or femoral lymph nodes, rarely affecting more than one. Other locations have been described as well – 4 cases of lymph node involvement in the pelvis, only 1 in women [610], 3 cases of cervical lymph node involvement [4, 11, 12], 2 cases of post-auricular lymph node involvement, both in men [13, 14] and 3 cases in men with paraaortic and/or pelvic lymph node involvement [2, 15, 16]. The pathogenesis of this disease is unclear, suggesting that it may be a secondary response to inflammation of the lymph nodes responding to vascular proliferation [9].

The disease is usually asymptomatic only with swelling of the relevant lymph node, except for the popliteal or post-auricular lymph nodes. In these cases, a pain syndrome can be observed [8, 13]. In a case involving the paraaortic lymph nodes, nausea and reflux were observed [2]. Angiomyomatous hamartoma of the lymph node may cause swelling of the affected limb [3, 20, 22, 30, 31].

Table 1 presents the clinical characteristics of all women with this disease described in the literature.

Table 1

Clinical characteristics of women with angiomyomatous hamartoma of the lymph node described in the literature

CaseAuthorYear of publicationAgeRegionSymptoms
1Chan, et al. [3]199210Left inguinalLeft inguinal mass
2Chan, et al. [3]199250Right inguinalRight inguinal mass
3Allen, et al. [17]199367Right femoralRight femoral mass
4Laeng, et al. [5]199617Right cervicalRight cervical mass
5Magro, et al. [18]199734Left inguinalLeft inguinal mass
6Süllü, et al. [19]200533Right inguinalRight inguinal mass
7Piedimonte, et al. [20]200634Left inguinalLeft inguinal mass
8Adaime, et al. [21]200974Right inguinalRight inguinal mass
9Bourgeois, et al. [22]200915Left inguinalLeft inguinal mass
10Barzilai, et al. [4]200951Left submandibularLeft submandibular mass
11Kim, et al. [6]201137Right poplitealSwelling and pain in the right knee
12Dzombeta, et al. [9]201267Right inguinalRight inguinal mass
13Catania, et al. [11]20128 monthsMidline cervicalMidline cervical mass
14Ding, et al. [23]2014N/AN/AN/A
15Lee, et al. [24]201589Right inguinalRight inguinal mass
16Plantinga, et al. [16]201545Pelvic lymph nodesN/A
17Arava, et al. [25]201670Right inguinalRight inguinal mass
18Arava, et al. [25]201622Right inguinalRight inguinal mass
19Arava, et al. [25]201624Right inguinalRight inguinal mass
20Moh, et al. [26]2017N/AN/AN/A
21Moh, et al. [26]2017N/AN/AN/A
22Moh, et al. [26]2017N/AN/AN/A
23Moh, et al. [26]2017N/AN/AN/A
24Moh, et al. [26]2017N/AN/AN/A
25Moh, et al. [26]2017N/AN/AN/A
26Moh, et al. [26]2017N/AN/AN/A
27Moh, et al. [26]2017N/AN/AN/A
28Woolley, et al. [27]201959Left inguinalPain
29Pyakurel, et al. [28]201912Right inguinalRight inguinal mass
30Xu, et al. [29]201957Left inguinalLeft inguinal mass
31Present case202137Paraaortic and pelvic lymph nodesWeight loss, pain

[i] N/A – not applicable

Angiomyomatous hamartoma of the lymph node can affect people at any age but is considered to most commonly affect people in their 60s [26]. The youngest patient reported was 8 months old with an enlarged cervical lymph node [12]. The disease usually persists for years and does not recur after resection of the affected lymph node. Only one exception of recurrence has been described in the literature – pain in the left inguinal area confirmed a recurrence 14 years after AMH-LN excision [27]. The patient was treated conservatively. The optimal treatment of AMH-LN is total resection of all enlarged lymph nodes.

The differential diagnosis of AMH includes lymphangiomyomatosis, which, unlike AMH, involves primarily thoracic and intra-abdominal lymph nodes: nodal leiomyomatosis with less pronounced vascular proliferation and angiomyolipoma of the lymph node. The latter is composed of the same tissues as in AMH, but the smooth muscle component shows increased cellularity, polymorphism and increased mitotic activity, as well as a typical immune profile with coexpression of melanocyte markers and estrogen, which were negative in our case.

In our case, the disease was manifested by weight loss and moderate pelvic pain with no evidence of inflammatory disease. The process involved pelvic and paraaortic nodes, which is not typical of AMH-LN. We did not perform a total resection of all enlarged lymph nodes during the surgery as we did not have a definitive diagnosis. The cryosection material did not provide information about the process. This forced us to stop the surgical intervention and wait for the permanent preparation.

Conclusions

Angiomyomatous hamartoma of the lymph node is a rare disease without classic clinical symptoms. The world literature references show that this is the first reported case in which the disease manifested itself with weight loss and affected paraaortic lymph nodes in a female patient.

Disclosure

The authors report no conflict of interest.

References

1 

Chaaya G, Kang L, Vishnubhotla P. A typical angiomyomatous hamartoma with unexplained hepatosplenomegaly. Fed Pract 2016; 33: 38-40.

2 

Jobbagy Z, Guerrieri C, Savatta D, Chattu S, Fyfe-Kirschner B. A rare case of angiomyomatous hamartoma of periaortic lymph node and synchronous angiomyolipoma of the kidney: a diagnostic pitfall for lymph node involvement by angiomyolipoma. Hum Pathol Case Rep 2020; 19: 200361.

3 

Chan JK, Frizzera G, Fletcher CD, Rosai J. Primary vascular tumors of lymph nodes other than Kaposi’s sarcoma. Analysis of 39 cases and delineation of two new entities. Am J Surg Pathol 1992; 16: 335-350.

4 

Barzilai G, Schindler Y, Cohen-Kerem R. Angiomyomatous hamartoma in a submandibular lymph node: a case report. Ear Nose Throat J 2009; 88: 831-832.

5 

Laeng RH, Hotz MA, Borisch B. Angiomyomatous hamartoma of a cervical lymph node combined with haemangiomatoids and vascular transformation of sinuses. Histopathology 1996; 29: 80-84.

6 

Kim HS, Na KY, Lee JH, Cho NS, Kim GY, Lim SJ. Angiomyomatous hamartoma of popliteal lymph nodes occurring in association with diffuse pigmented villonodular synovitis of knee. J Pathol Transl Med 2011; 45: S58-S61.

7 

Prusac IK, Juric I, Lamovec J, Culic V. Angiomyomatous hamartoma of the popliteal lymph nodes in a patient with Klippel-Trenaunay syndrome: case report. Fetal Pediatr Pathol 2011; 30: 320-324.

8 

Mauro CS, McGough RL, Rao UN. Angiomyomatous hamartoma of a popliteal lymph node: an unusual cause of posterior knee pain. Ann Diagn Pathol 2008; 12: 372-374.

9 

Dzombeta T, Francina M, Matković K, et al. Angiomyolipomatous hamartoma of the inguinal lymph node–report of two cases and literature review. In Vivo 2012; 26: 459-462.

10 

Mridha AR, Ranjan R, Kinra P, Ray R, Khan SA, Shivanand G. Angiomyomatous hamartoma of popliteal lymph node: an unusual entity. J Pathol Transl Med 2015; 49: 156-158.

11 

Catania VD, Manzoni C, Novello M, Lauriola L, Coli A. Unusual presentation of angiomyomatous hamartoma in an eight-month-old infant: case report and literature review. BMC Pediatr 2012; 12: 172.

12 

Balci MG, Tayfur M. A rare lesion of cervical lymph nodes: angiomyomatous hamartoma. Med Sci Discov 2021; 8: 187-188.

13 

Gamsizkan M, Akkaya A. Angiomyomatous hamartoma of postauricular lymph nodes (case report). J Clin Analytic Med 2013; 4: 420-422.

14 

Karim MS, Ensslin CJ, Dowd ML, Samie FH. Angiomyomatous hamartoma in a postauricular lymph node: a rare entity masquerading as a cyst. JAAD Case Rep 2020; 7: 131-133.

15 

Mingrino J, Kurt H. Angiomyomatous hamartoma with extensive lymphadenopathy. Blood 2020; 136: 1794.

16 

Plantinga P, Chan N, Wehrli B, Sangle N. A case of angiomyomatous hamartoma in pelvic lymph nodes. Can J Pathol 2015; 7: 28-29.

17 

Allen PW, Hoffman GJ. Fat in angiomyomatous hamartoma of lymph node. Am J Surg Pathol 1993; 17: 748-749.

18 

Magro G, Grasso S. Angiomyomatous hamartoma of the lymph node: case report with adipose tissue component. Gen Diagn Pathol 1997; 143: 247-249.

19 

Süllü Y, Gun S, Dabak N, Karagoz F. Angiomyomatous hamartoma in the inguinal lymph node: a case report. Turk J Pathol 2006; 22: 42-44.

20 

Piedimonte A, De Nictolis M, Lorenzini P, Sperti V, Bertani A. Angiomyomatous hamartoma of inguinal lymph nodes. Plast Reconstr Surg 2006; 117: 714-716.

21 

Adaime SBR, Ddine CC, Londero TM. Hamartoma nodal angiomiomatoso. Rev AMRIGS 2009; 53: 72-74.

22 

Bourgeois P, Dargent JL, Larsimont D, et al. Lymphoscintigraphy in angiomyomatous hamartomas and primary lower limb lymphedema. Clin Nucl Med 2009; 34: 405-409.

23 

Ding Q, Hammer R. Angiomyomatous hamartoma of lymph nodes is associated with dermatopathic lymphadenopathy. Am J Clin Pathol 2014; 142: A086.

24 

Lee CH, Chang TC, Ku JW. Angiomyomatous hamartoma in an inguinal lymph node with proliferating pericytes/smooth muscle cells, plexiform vessel tangles, and ectopic calcification. Indian J Pathol Microbiol 2015; 58: 226-228.

25 

Arava S, Gahlot GP, Deepak R, Sharma MC, Nath D, Ashok S. Angiomyomatous hamartoma of lymph nodes: clinicopathological study of 6 cases with review of literature. Indian J Pathol Microbiol 2016; 59: 206-208.

26 

Moh M, Sangoi AR, Rabban JT. Angiomyomatous hamartoma of lymph nodes, revisited: clinicopathologic study of 21 cases, emphasizing its distinction from lymphangioleiomyomatosis of lymph nodes. Hum Pathol 2017; 68: 175-183.

27 

Woolley CA, Oswald J, Chen J. Painful inguinal angiomyomatous hamartoma responsive to conservative pain management. A A Pract 2019; 13: 373-375.

28 

Pyakurel D, Gautam K, Shrestha A, Pradhan S, Pant V. Angiomyomatous hamartoma in an inguinal lymph node. J Pediatr Surg Case Rep 2019; 43: 42-43.

29 

Xu H, Feng X, Lockhart V, Cotelingam J, Veillon D, Shi M. Angiomyomatous hamartoma in the inguinal lymph node: a case report and literature review. Hum Pathol Case Rep 2020; 19: 200351.

30 

Ram M, Alsanjari N, Ansari N. Angiomyomatous hamartoma: a rare case report with review of the literature. Rare Tumors 2009; 1: e25.

31 

Dargent JL, Lespagnard L, Verdebout JM, Bourgeois P, Munck D. Glomeruloid microvascular proliferation in angiomyomatous hamartoma of the lymph node. Virchows Arch 2004; 445: 320-322.

Copyright: © 2023 Termedia Sp. z o. o. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (http://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
 
Quick links
© 2024 Termedia Sp. z o.o.
Developed by Bentus.