Background

This anatomic region is largely ignored in medicine. Yet, it can be the site of varied and sometimes devastating diseases. The following outlines various pathologies which may present with an intrascrotal mass.

OUTLINE

Clinical and Histologic Variants	CYSTS, HYPERPLASIAS, AND NEOPLASMS OF THE TESTICULAR COLLECTING SYSTEM HYPERPLASIAS AND NEOPLASMS OF THE PARATESTICULAR MESENCHYME MISCELLANEOUS RARE PRIMARY NEOPLASMS AND METASTATIC DISEASE TUMOR-LIKE CONDITIONS
Commonly Used Terms
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CYSTS, HYPERPLASIAS, AND NEOPLASMS OF THE TESTICULAR COLLECTING SYSTEM

Lesions of the Rete Testis
Non-neoplastic and developmental abnormalities	Rete hyperplasia Nodular proliferation of calcifying connective tissue Cystic dysplasia Acquired or secondary cystic changes in the rete Pathology of the rete in cryptorchidism
Benign neoplasms	Cystadenoma of usual type Sertoliform cystadenoma
Lesions of the Epididymis	Cysts of the epididymis Papillary cystadenoma of the epididymis Carcinoma of the epididymis
Metastatic Tumors

CYSTS, HYPERPLASIAS, AND NEOPLASMS OF THE PARATESTICULAR MESOTHELIUM

Cysts	Mesothelioma cyst Reactive mesothelioma hyperplasia
Benign Neoplasms	Adenomatoid tumor Benign cystic mesothelioma Well-differentiated papillary mesothelioma
Malignant Neoplasms	Malignant mesothelioma
OTHER
Paratesticular cysts with benign epithelial proliferations of wolffian origin. Nistal M, Gonzalez-Peramato P, Serrano A, Vega-Perez M, De Miguel MP, Regadera J. Department of Pathology, La Paz University Hospital, Madrid and the Department of Anatomy, Histology and Neuroscience, School of Medicine, Autonomous University of Madrid.	Am J Clin Pathol. 2005 Aug;124(2):245-51. Abstract quote   Paratesticular cysts with benign epithelial proliferations (BEPs) are rare. Only 10 cases were found in a series of 431 paratesticular cysts and were classified as follows: cystadenoma, 5; papilloma, 2; and hamartoma, 3. Four cystadenomas showed multiple papillae lined by CD10+ epithelial cells with hyperchromatic nuclei. The remaining lesion showed areas with a microcystic, glandular, cribriform pattern, with small, benign glands without atypia. Urothelial papilloma presented BEPs with cytokeratin (CK) 7+ and CD10+ and CK20- umbrella-like cells. The mural papilloma was lined by proliferative cylindrical cells exhibiting strong CK7 and CD10 expression. The 3 Wolffian hamartomas were characterized by strongly CD10+ epithelium surrounded by smooth muscle cells. The consistent CD10 expression in BEPs of paratesticular cysts suggests a Wolffian origin. The differential diagnosis of paratesticular cysts with BEP vs metastatic prostatic and primary borderline or malignant tumors is discussed.

HYPERPLASIAS AND NEOPLASMS OF THE PARATESTICULAR MESENCHYME

Benign Tumors	Aggressive angiomyxoma Angiomyofibroblastoma Lipoma
Malignant Tumors	Liposarcoma Malignant fibrous histiocytoma Leiomyosarcoma Rhabdomyosarcoma Desmoplastic round cell tumor Melanotic neuroectodermal tumor of infancy

MISCELLANEOUS RARE PRIMARY NEOPLASMS AND METASTATIC DISEASE

Ovarian-type Tumors	Serous Mucinous Endometrioid Extratesticular sex-cord stromal tumors Dermoid cysts
Serous Borderline Tumor of the Paratestis A Report of Seven Cases	Am J Surg Pathol 2001;25:373-378 We report the clinical, morphologic, immunophenotypic, and ploidy findings of seven cases of serous borderline tumor of the paratestis. Mean patient age was 56 years (range, 14–77 years), and the clinical presentation was that of a testicular mass. Tumors ranged in size from 1 to 6 cm (mean, 3.5 cm). Six tumors arose from the tunica albuginea, and two of these tumors were intratesticular. One tumor arose from the tunica vaginalis. Serous borderline tumor of the paratestis is histologically identical to its ovarian counterpart. The tumors were cystic with numerous intracystic blunt papillae lined by stratified epithelial cells with minimal to mild cytologic atypia. Psammoma bodies were present in two cases. In all cases, the neoplastic cells stained strongly and diffusely for cytokeratin 7, estrogen receptor, and CD15, and six of seven cases were positive for progesterone receptor and MOC-31. The cells did not stain for cytokeratin 20, carcinoembryonic antigen, calretinin, and HER2/neu. Proliferative activity, as assessed by MIB-1 staining, ranged from 1.3% to 10% (mean, 5.5%). Five of six tumors were diploid, and one was tetraploid. Patients were treated by radical orchiectomy and followed up from 4 months to 18 years (mean, 48 months; median, 8.5 months). No recurrences or metastases occurred. Conclusion: Serous borderline tumor of the paratestis is morphologically and immunophenotypically identical to ovarian serous borderline tumor. To date, no serous borderline tumor of the paratestis reported in the literature or in our series has recurred or metastasized after resection.
	Am J Surg Pathol 1988;12:56–63.
Paratesticular serous papillary carcinoma: a report of six cases	Am J Surg Pathol 1995;19:1359–65
Other	Extrarenal Wilm's tumor Ectopic adrenal tissue Neuroblastoma Paraganglioma
Hematopoietic	Lymphoma Plasmacytoma Granulocytic sarcoma
Metastatic Tumors	Stomach Kidney Prostate

TUMOR-LIKE CONDITIONS

Aspects of normal histology	Cribriform pattern of epididymis Bizarre nuclear atypia within epithelial cells of epididymis Extraparenchymal Leydig cells
Hydrocele-related changes
Nongranulomatous epididymitis
Granulomatous epididymitis	Infectious Idiopathic
Malakoplakia
Sarcoidosis
Changes associated with vasculitis
Inflammatory pseudotumor
Spermatocele
Sperm granuloma and other granulomatous processes
Vasitis nodosa/epididymitis nodosa
Fibrous pseudotumor
Meconium periorchitis
Mesothelial hyperplasia
Tumor of the adrenogenital syndrome and related lesions
Sclerosing lipogranuloma
Splenic-gonadal fusion
Fibromuscular hyperplasia
Granulomatous lymphangitis of the scrotum and penis Report of a case and review of the literature of genital swelling with sarcoidal granulomatous inflammation Michael J. Murphy1, Barry Kogan2 and J. Andrew Carlson3 1 Department of Dermatology, University of Connecticut Health Center, Farmington, Connecticut, USA,2 Division of Urology, Albany Medical College, Albany, New York, USA,3 Divisions of Dermatology and Dermatopathology, Albany Medical College, Albany, New York, USA	Journal of Cutaneous Pathology 2001;28 (8), 419-424 Abstract quote Background: Acquired lymphedema of the genitalia is a rare childhood presentation and is more common in elderly individuals secondary to pelvic/abdomenal malignancy or its therapy or worldwide due to filariasis. Objective: Herein, we report a case of a healthy 11-year-old boy who presented with a 1-year history of chronic, asymptomatic scrotal and penile swelling. Biopsy revealed edema, lymphangiectases and peri- and intralymphatic sarcoidal type granulomas. This histologic pattern of granulomatous lymphangitis is most commonly associated with orofacial granulomatosis (granulomatous cheilitis and Melkersson-Rosenthal syndrome) and Crohn’s disease. Treatment with topical steroids and physical support has resulted in marked improvement. No systemic disease (Crohn’s disease) is evident 1 year later. Literature review revealed 44 cases of genital lymphedema with non-infectious granulomas. The majority of these young patients had Crohn’s disease, frequently with anal involvement and a minority, both with and without Crohn’s disease, had orofacial granulomatosis. Conclusions: Granulomatous lymphangitis should be considered in the differential diagnosis of chronic idiopathic swelling of the genitalia, particularly in younger individuals. Further clinical examination, additional laboratory studies and close follow-up for co-existing or subsequent development of Crohn’s disease should be performed. The overlap between granulomatous lymphangitis of the genitalia, Crohn’s disease and orofacial granulomatosis suggest that granulomatous lymphangitis of the genitalia may represent a forme fruste of Crohn’s disease.
FIBROUS PSEUDOTUMOR OF THE TESTICULAR TUNICS
Diffuse fibrous pseudotumor of the testicular tunics associated with an inflamed hydrocele. Seethala RR, Tirkes AT, Weinstein S, Tomaszewski JE, Malkowicz SB, Genega EM. Departments of Pathology and Laboratory Medicine (Drs Seethala, Tomaszewski, and Genega), Radiology (Drs Tirkes and Weinstein), and Urology (Dr Malkowicz), Hospital of the University of Pennsylvania, Philadelphia.	Arch Pathol Lab Med 2003 Jun;127(6):742-4 Related Articles, Links Diffuse fibrous pseudotumor of the testicular tunics associated with an inflamed hydrocele. Seethala RR, Tirkes AT, Weinstein S, Tomaszewski JE, Malkowicz SB, Genega EM. Departments of Pathology and Laboratory Medicine (Drs Seethala, Tomaszewski, and Genega), Radiology (Drs Tirkes and Weinstein), and Urology (Dr Malkowicz), Hospital of the University of Pennsylvania, Philadelphia. Fibrous pseudotumors of the testicular tunics and paratesticular soft tissue are uncommon lesions. They typically arise as painless scrotal masses that may be associated with a hydrocele or history of trauma or infection. Although these lesions are clinically worrisome for a malignant neoplasm, they are thought to be reactive in nature, since they are composed of dense fibrous tissue with interspersed bland fibroblasts and myofibroblasts and mixed inflammatory cells. Once excised, these lesions behave in a benign fashion. Typically, these masses are multinodular, but in rare cases they are diffuse, bandlike myofibroblastic proliferations that encase the testis and are termed fibromatous periorchitis. Herein, we describe a 57-year-old man with a diffuse fibrous pseudotumor/fibromatous periorchitis that encased the left testis and adnexa and arose in close apposition to an inflamed hydrocele.
A rare scrotal mass: fibrous pseudotumor of epididymis. Gogus O, Bulay O, Yurdakul T, Beduk Y. Department of Urology, Ankara University ibn-i Sina (Avicenna) Hospital, Turkey.	Urol Int 1990;45(1):63-4 Abstract quote Fibrous pseudotumor, also called multiple fibromata pseudofibromatous periorchitis, is a rare testicular fibromatous condition. It is a benign fibroproliferative lesion with dense hyalinization and sometimes focal calcification. Most of the cases occur in the testicular tunics, whereas a few originate from the epididymis.
A rare scrotal mass: fibrous pseudotumor of epididymis. Gogus O, Bulay O, Yurdakul T, Beduk Y. Department of Urology, Ankara University ibn-i Sina (Avicenna) Hospital, Turkey.	Urol Int 1990;45(1):63-4 Abstract quote Fibrous pseudotumor, also called multiple fibromata pseudofibromatous periorchitis, is a rare testicular fibromatous condition. It is a benign fibroproliferative lesion with dense hyalinization and sometimes focal calcification. Most of the cases occur in the testicular tunics, whereas a few originate from the epididymis.
Immunohistological studies of fibrous pseudotumor of the testicular tunica vaginalis. Takebayashi K, Fujioka Y, Nagashima K, Nagakawa T, Kubota M. Department of Pathology, Hokkaido University School of Medicine, Sapporo, Japan.	Acta Pathol Jpn 1989 Dec;39(12):810-3 Abstract quote A fibrous pseudotumor of the testis from a 46-year-old man was examined histologically and immunohistochemically. The tumor, situated in the tunica vaginalis, appeared as multiple round nodules up to 2 cm in diameter, and was composed largely of fibrous tissue with scanty inflammatory cell infiltration. Besides mesothelial cells covering part of the tumor, foci of piled-up cells were found among the fibrous tumor tissue. The cells in the foci were spindle and polygonal in shape, and contained oval nuclei. Immunohistochemically, these cells were positive for vimentin and keratin, but negative for CEA. Similar features have been found in pleural mesothelioma, supporting the concept that testicular fibrous pseudotumor could represent neoplastic growth of the mesothelium of the tunica vaginalis.
Fibrous pseudotumor of the tunica vaginalis testis. Bruijnes E, Ladde BE, Dabhoiwala NF, Stukart RA.	Urol Int 1984;39(5):314-7 Abstract quote The incidence of fibrous pseudotumor of the tunica vaginalis testis is rare. It is a benign, fibroproliferative process with hyalinization and sometimes with focal calcification. In most cases malignancy is suspected although by careful physical examination it may be possible to establish the diagnosis pre-operatively. The best form of therapy is frozen section biopsy of the pseudotumor followed by excision; including if necessary the tunica vaginalis. If the pseudotumor is locally extensive an epididymectomy is sometimes necessary. A study of the literature reveals that because of the rarity of this lesion orchidectomy appears to have been performed in most cases. A case report of a patient with fibrous pseudotumor of the tunica vaginalis testis is presented.
SCROTAL CALCINOSIS
SMOOTH MUSCLE HAMARTOMA OF THE SCROTUM
Acquired smooth-muscle hamartoma of the scrotum: a histological simulator? Van Kooten EO, Hage JJ, Meinhardt W, Horenblas S, Mooi WJ. Department of Plastic and Reconstructive Surgery, Netherlands Cancer Institute/Antoni van Leeuwenhoek Hospital, Amsterdam, The Netherlands.	J Cutan Pathol. 2004 May;31(5):388-92. Abstract quote   Background: In three cases of chronic scrotal lymphedema, histological and immunohistochemical changes were observed that were strikingly similar to an exceedingly rare lesion reported previously under the name of acquired smooth-muscle hamartoma (ASMH) of the scrotum. The clinical context indicated that the cases were reactive rather than hamartomatous in nature. Materials and methods: The histological and immunohistochemical findings of the three cases were compared to macroscopically normal scrotal specimens obtained during sex reassignment surgery in seven male-to-female transsexuals. Results: Compared to the seven controls, the three cases of chronic scrotal edema revealed a marked increase of dartos smooth-muscle tissue and of connective tissue of the scrotal skin and underlying soft tissues. Still, even the normal amount of scrotal smooth-muscle tissue may easily be misinterpreted as smooth-muscle hyperplasia. Conclusions: Chronic scrotal lymphedema may induce hyperplasia of the dartos muscle, resulting in a histological appearance previously described as ASMH. This indicates that ASMH may not always represent a later onset of abnormality similar to congenital smooth-muscle hamartoma but, rather, may constitute a histological simulator.

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